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acquired von willebrand disease treatment

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https://www.readbyqxmd.com/read/28987857/intracranial-hemorrhage-in-patients-with-durable-mechanical-circulatory-support-devices-institutional-review-and-proposed-treatment-algorithm
#1
Wyatt L Ramey, Robyn L Basken, Christina M Walter, Zain Khalpey, G Michael Lemole, Travis M Dumont
OBJECTIVE: Spontaneous intracranial hemorrhage (ICH) is frequently managed in neurosurgery. Patients with durable mechanical circulatory support devices, including total artificial hearts (TAHs) and left ventricular assist devices (LVADs) are often encountered in the setting of ICH. While durable mechanical circulatory support devices have improved survival and quality of life for patients with advanced heart failure, ICH is one of the most feared complications following LVAD and TAH implantation...
October 4, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28759473/novel-therapeutic-approaches-for-thrombotic-thrombocytopenic-purpura
#2
Yvette C Tanhehco, Gowthami Arepally, Ara Metjian
PURPOSE OF REVIEW: Acquired thrombotic thrombocytopenic purpura is an immune-mediated thrombotic microangiopathy caused by antibodies to ADAMTS13 (A Disintegrin And Metalloproteinase with a ThromboSpondin type 1 motif, member 13). Standard treatment with therapeutic plasma exchange and immunosuppression with steroids results in high remission and low mortality rates. However, a number of patients remain refractory to frontline therapy and/or experience multiple relapses. This study reviews emerging therapies for thrombotic thrombocytopenic purpura...
November 2017: Current Opinion in Hematology
https://www.readbyqxmd.com/read/28645643/management-of-thrombotic-thrombocytopenic-purpura
#3
P Coppo
Daily therapeutic plasma exchange (TPE) transformed the historically fatal prognosis of acquired, anti-ADAMTS13 antibody-mediated thrombotic thrombocytopenic purpura (TTP), leading to the current overall survival rates of >80%. However, relapses occur in up to 40% of patients and refractory disease with fatal outcomes still occurs, typically within the first days of management. In this context, the introduction of rituximab has been the second major breakthrough in TTP management. Rituximab is now routinely recommended during the acute phase, typically in patients with a suboptimal response to treatment, and increasingly as frontline therapy, with high response rates in the following weeks...
September 2017: Transfusion Clinique et Biologique: Journal de la Société Française de Transfusion Sanguine
https://www.readbyqxmd.com/read/28554655/hemorrhagic-angiodysplasia-of-the-digestive-tract-pathogenesis-diagnosis-and-management
#4
REVIEW
Aymeric Becq, Gabriel Rahmi, Guillaume Perrod, Christophe Cellier
No abstract text is available yet for this article.
November 2017: Gastrointestinal Endoscopy
https://www.readbyqxmd.com/read/28512017/a-case-of-autoimmune-severe-acquired-von-willebrand-syndrome-type-3-like
#5
Chakri Gavva, Prapti Patel, Yu-Min Shen, Eugene Frenkel, Ravi Sarode
Von Willebrand disease (VWD) is the most common congenital bleeding disorder and is due to quantitative or qualitative defects of von Willebrand factor (VWF). Acquired defects of VWF, termed acquired von Willebrand syndrome (AVWS), are due to a host of different mechanisms. Autoantibody-mediated AVWS may be associated with lymphoproliferative or immunological disorders, such as systemic lupus erythematosus (SLE). A large majority of AVWS cases are type 1 or type 2A-like and patients tend to have a mild to moderate bleeding tendency...
June 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28480350/therapeutic-efficacy-of-the-platelet-glycoprotein-ib-antagonist-anfibatide-in-murine-models-of-thrombotic-thrombocytopenic-purpura
#6
Liang Zheng, Yingying Mao, Mohammad S Abdelgawwad, Nicole K Kocher, Mandy Li, Xiangrong Dai, Benjamin Li, X Long Zheng
Thrombotic thrombocytopenic purpura (TTP), a potentially fatal blood clot disorder, is primarily caused by severe deficiency of plasma ADAMTS13 activity resulting from acquired autoantibodies. Plasma exchange is the only effective initial therapy. However, the high mortality rate and the complications associated with plasma exchange therapy remain a major concern. To address unmet clinical needs, therapeutic efficacies of anfibatide, a snake venom-derived platelet glycoprotein Ib antagonist, in murine models of spontaneous thrombocytopenia and shigatoxin-induced TTP were determined...
November 29, 2016: Blood Advances
https://www.readbyqxmd.com/read/28416507/thrombotic-thrombocytopenic-purpura
#7
REVIEW
Bérangère S Joly, Paul Coppo, Agnès Veyradier
Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and organ ischemia linked to disseminated microvascular platelet rich-thrombi. TTP is specifically related to a severe deficiency in ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13), the specific von Willebrand factor-cleaving protease. ADAMTS13 deficiency is most frequently acquired via ADAMTS13 autoantibodies, but rarely, it is inherited via mutations of the ADAMTS13 gene...
May 25, 2017: Blood
https://www.readbyqxmd.com/read/28296884/increased-von-willebrand-factor-decreased-adamts13-and-thrombocytopenia-in-melioidosis
#8
Emma Birnie, Gavin C K W Koh, Ester C Löwenberg, Joost C M Meijers, Rapeephan R Maude, Nicholas P J Day, Sharon J Peacock, Tom van der Poll, W Joost Wiersinga
BACKGROUND: Melioidosis, caused by bioterror treat agent Burkholderia pseudomallei, is an important cause of community-acquired Gram-negative sepsis in Southeast Asia and Northern Australia. New insights into the pathogenesis of melioidosis may help improve treatment and decrease mortality rates from this dreadful disease. We hypothesized that changes in Von Willebrand factor (VWF) function should occur in melioidosis, based on the presence of endothelial stimulation by endotoxin, pro-inflammatory cytokines and thrombin in melioidosis, and investigated whether this impacted on outcome...
March 2017: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/28236847/is-von-willebrand-disease-linked-to-cholesteatoma-aetiology
#9
 Reis Rego, M Santos, M Coutinho, T Feliciano, C Almeida E Sousa
Cholesteatoma is a common clinical picture seen by otolaryngologists. The disease is characterised by an abnormal grow "of skin in the wrong place". Specifically, in acquired cholesteatoma, the main causative issue is associated with ventilatory deficits within the middle ear and results in chronic infection. Molecular science has associated the presence of specific molecules with its development, and moreover recent research suggests that deregulated angiogenesis is a crucial process in the development of cholesteatoma and its recurrence...
March 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/28110841/treatment-of-autoimmune-thrombotic-thrombocytopenic-purpura-in-the-more-severe-forms
#10
REVIEW
Paul Coppo
Daily therapeutic plasma exchange (TPE) transformed the historically fatal prognosis of acquired, anti-ADAMTS13 antibody-mediated thrombotic thrombocytopenic purpura (TTP), leading to the current overall survival rates of >80%. However, relapses occur in up to 40% of patients and refractory disease with fatal outcomes still occurs. In this context, the introduction of rituximab has probably been the second major breakthrough in TTP management. Rituximab is now routinely recommended during the acute phase, typically in patients with a suboptimal response to treatment, or even as frontline therapy, with high response rates...
February 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/27991718/polycythemia-vera-and-essential-thrombocythemia-2017-update-on-diagnosis-risk-stratification-and-management
#11
Ayalew Tefferi, Tiziano Barbui
DISEASE OVERVIEW: Polycythemia Vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms respectively characterized by erythrocytosis and thrombocytosis; other disease features include leukocytosis, splenomegaly, thrombosis, bleeding, microcirculatory symptoms, pruritus, and risk of leukemic or fibrotic transformation. DIAGNOSIS: PV is defined by a JAK2 mutation, whose absence, combined with normal or increased serum erythropoietin level, makes the diagnosis unlikely...
January 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/27834763/acquired-von-willebrand-syndrome-an-under-recognized-cause-of-major-bleeding-in-the-cardiac-intensive-care-unit
#12
Melissa B Jones, Karthik Ramakrishnan, Fahad A Alfares, Kendal M Endicott, Gary Oldenburg, John T Berger, Venkat Shankar, Dilip S Nath, Yaser A Diab
BACKGROUND: Acquired von Willebrand syndrome (AvWS) in the setting of congenital heart disease is an under-recognized cause of bleeding in the pediatric cardiac critical care unit. METHODS: Fourteen patients diagnosed with AvWS admitted to the cardiac intensive care unit at the Children's National Health System between December 2009 and September 2015 were identified with subsequent chart review and case analysis. RESULTS: Of the 14 patients included in this study, 4 patients were on ventricular-assist devices, 6 patients were on extracorporeal membrane oxygenation, and 4 were patients with congenital heart disease not receiving any mechanical circulatory support...
November 2016: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/27587252/-acquired-von-willebrand-syndrome-in-three-patients-and-literature-review
#13
REVIEW
H Y Ouyang, Z J Yu, J Yin, X J Zhao, Z Y Wang, W Zhang, Z N Ma, J Su, X Bai, C G Ruan
OBJECTIVE: To deepen the understanding of acquired von Willebrand syndrome (AVWS). METHODS: The clinical data of 3 patients were analyzed and related literature were reviewed. RESULTS: ① Case 1, a 70- year- old male, diagnosed as Waldenstrom macroglobulinemia and AVWS, was presented with spontaneous epitaxis and bruising. The VWF∶Ag level was 16%. Treatment was initiated with VWF concentrates. Two cycles of chemotherapy with Bortezomib, thalidomide and Dexamethasone were followed...
August 14, 2016: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/27479501/generation-of-anti-murine-adamts13-antibodies-and-their-application-in-a-mouse-model-for-acquired-thrombotic-thrombocytopenic-purpura
#14
Louis Deforche, Claudia Tersteeg, Elien Roose, Aline Vandenbulcke, Nele Vandeputte, Inge Pareyn, Elien De Cock, Hanspeter Rottensteiner, Hans Deckmyn, Simon F De Meyer, Karen Vanhoorelbeke
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening thrombotic microangiopathy linked to a deficiency in the metalloprotease ADAMTS13. In the current study, a novel mouse model for acquired TTP was generated to facilitate development and validation of new therapies for this disease. Therefore, a large panel (n = 19) of novel anti-mouse ADAMTS13 (mADAMTS13) monoclonal antibodies (mAbs) of mouse origin was generated. Inhibitory anti-mADAMTS13 mAbs were identified using the FRETS-VWF73 assay. Four mAbs strongly inhibited mADAMTS13 activity in vitro (∼68-90% inhibition)...
2016: PloS One
https://www.readbyqxmd.com/read/27276895/a-case-of-hypertrophic-obstructive-cardiomyopathy-and-acquired-von-willebrand-syndrome-response-to-medical-therapy
#15
Masaki Wake, Nobuyuki Takahashi, Hiroyuki Yoshitomi, Kazuaki Tanabe
Heyde's syndrome is the combined occurrence of acquired von Willebrand disease caused by aortic valve stenosis and gastrointestinal bleeding that occurs particularly in elderly patients. The bleeding may be linked to the intravascular shear-induced proteolysis of high-molecular-weight multimers (HMWMs) of von Willebrand factor (vWF). Hypertrophic obstructive cardiomyopathy (HOCM) in the left ventricular outflow tract generates a high shear stress condition that can induce such proteolysis. We report the case of a 70-year-old woman with HOCM who had severe anemia and loss of HMWMs...
September 2014: Journal of Echocardiography
https://www.readbyqxmd.com/read/27040023/adamts13-autoantibodies-cloned-from-patients-with-acquired-thrombotic-thrombocytopenic-purpura-2-pathogenicity-in-an-animal-model
#16
Eric M Ostertag, Khalil Bdeir, Stephen Kacir, Michelle Thiboutot, Gayathri Gulendran, Lenka Yunk, Vincent M Hayes, David G Motto, Mortimer Poncz, X Long Zheng, Douglas B Cines, Don L Siegel
BACKGROUND: Acquired thrombotic thrombocytopenic purpura (TTP) is a potentially fatal disease in which ultralarge von Willebrand factor (UL-VWF) multimers accumulate as a result of autoantibody inhibition of the VWF protease, ADAMTS13. Current treatment is not specifically directed at the responsible autoantibodies and in some cases is ineffective or of transient benefit. More rational, reliable, and durable therapies are needed, and a human autoantibody-mediated animal model would be useful for their development...
July 2016: Transfusion
https://www.readbyqxmd.com/read/26838696/treatment-of-von-willebrand-disease
#17
REVIEW
Jennifer Curnow, Leonardo Pasalic, Emmanuel J Favaloro
Congenital von Willebrand disease (VWD) and acquired von Willebrand syndrome (AVWS) reflect conditions caused by von Willebrand factor (VWF) deficiency and/or defects. VWD is the most common inherited bleeding disorder and AVWS arises from a variety of causes. Since VWF stabilizes and protects factor VIII (FVIII) in the circulation, this is also reduced in many patients with VWD. The treatment of VWD and AVWS therefore primarily entails replacement of VWF, and sometimes FVIII, to protect against bleeding. This may entail the use of VWF concentrates (currently plasma-derived) and/or FVIII concentrates (currently plasma-derived or more increasingly recombinant forms), and/or desmopressin to release endogenous VWF in subgroups of patients...
March 2016: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/26771972/acquired-von-willebrand-syndrome-during-the-course-of-myelofibrosis-analysis-of-32-cases
#18
Andrzej Mital, Witold Prejzner, Andrzej Hellmann
BACKGROUND: Identification of patients with myelofibrosis being at increased risk of acquired von Willebrand syndrome (avWS) would likely facilitate individualization of treatment and improve its outcomes. OBJECTIVES: To determine the prevalence of avWS in patients with myelofibrosis, and to verify if individuals with and without this bleeding disorder differ in terms of their baseline clinical parameters. MATERIAL AND METHODS: The study included 32 consecutive patients with myelofibrosis...
November 2015: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
https://www.readbyqxmd.com/read/26637782/treatment-of-thrombotic-thrombocytopenic-purpura-beyond-therapeutic-plasma-exchange
#19
REVIEW
Paul Coppo, Antoine Froissart
Daily therapeutic plasma exchange (TPE) transformed the historically fatal prognosis of acquired, anti-ADAMTS13 antibody-mediated thrombotic thrombocytopenic purpura (TTP), leading to the current overall survival rates of 80%-85%. However, relapses occur in ~40% of patients and refractory disease with fatal outcomes still occurs. In this context, the introduction of rituximab has probably been the second major breakthrough in TTP management. Rituximab is now routinely recommended during the acute phase, typically in patients with a suboptimal response to treatment, or even as frontline therapy, with high response rates...
2015: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/26637727/acquired-bleeding-disorders-in-the-elderly
#20
Rebecca Kruse-Jarres
The hemostatic balance changes with advancing age which may be due to factors such as platelet activation, increase of certain clotting factor proteins, slowing of the fibrinolytic system, and modification of the endothelium and blood flow. Generally, this predisposes the elderly to thrombosis rather than bleeding. It often necessitates antiplatelet or anticoagulation therapy, which can cause significant bleeding problems in an aging population. Additionally, changing renal function, modification in immune regulation, and a multitude of other disease processes, can give rise to acquired bleeding disorders...
2015: Hematology—the Education Program of the American Society of Hematology
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